Biomedical subjects
Publications and source records attributed to A M Karnik.
Pneumothorax can be spontaneous, traumatic or iatrogenic. Pneumothorax ex vacuo, sports-related pneumothorax and barotrauma unrelated to mechanical ventilation are interesting and newer entities. Management consists of getting rid of the air and prevention of recurrence of pneumothorax.
OBJECTIVE: To describe the presentation and genetic transmission of ciliary dyskinesia syndrome associated with hydrocephalus and mental retardation in 3 generations of a family. PATIENTS AND METHODS: A large Jordanian family included 9 individuals in 3 generations with recurrent pulmonary infections; 4 male siblings have been diagnosed as having mental retardation, and a maternal uncle was believed to have been similarly affected. Chromosome analysis of the family showed a normal karyotype. RESULTS: Electron microscopy of the nasal cilia from 3 affected siblings showed features of primary ciliary dyskinesia. Computed tomographic scans of the brains of all 4 affected siblings showed hydrocephalus. CONCLUSIONS: The recurrent pulmonary infections and hydrocephalus in this large Jordanian family are likely related to ciliary dyskinesia, which appears to follow an autosomal recessive mode of inheritance. The unusual presentation of ciliary dyskinesia, hydrocephalus, and mental retardation may be due to a new genetic mutation.
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Clinical diagnosis is a process of logical deduction from the data gathered by history and physical examination. When organic causes of an illness have been ruled out, a diagnosis of "functional disorder" or "conversion reaction" is considered. Cost of care of such patients can be enormous, especially when a large number of investigations are done to find an organic illness, which does not really exist. In such cases, a positive and early diagnosis of a conversion reaction can save needless tests and much distress to the patient. This report describes a case of paraplegia that was investigated for years before a diagnosis of conversion reaction was firmly made, based on a novel observation. We believe that we describe here a new physical sign, which can be used to diagnose "hysterical paraplegia."
Uncontrolled infection quite often leads to systemic inflammatory reaction syndrome (SIRS) and multiorgan dysfunction (MOD) syndrome. Thirty-five consecutive patients (19 males) fulfilling strict diagnostic criteria for SIRS were enrolled in two multicenter prospective double-blind trials involving new therapies for SIRS. The patients were followed prospectively up to day 28 after the enrollment. In the 35 patients with SIRS, males predominated in the age group below 40 (10/12, 83%) compared to the older group (nine males out of 23, 39%). Out of 16 females presenting with SIRS, only two were below the age of 40. This distribution was statistically different than our general MICU population. The serum albumin in these patients was uniformly low, with a mean of 22.5 gm/L. The bulk of SIRS patients (22/35; 63%) went on to develop acute renal failure (ARF). Although statistically not different, skin and peritoneal infections were more common in ARF group while pulmonary infections in non-ARF group. The majority of blood-cultures grew gram-positive organisms. Resolution of SIRS occurred within first 3 days in greater number of non-ARF survivors than ARF survivors (6/9, 66.7% vs. 6/16, 37.5%). Of the 22 ARF patients, 17 showed improvement in their renal function; the five who did not, died before day 28. The overall mortality (about 32%) was similar in both groups. Patients who developed both ARF and ARDS did not survive. In conclusion. SIRS occurs mostly in elderly patients, almost always in patients with low albumin levels. Premenopausal women seem to be protected. Blood cultures isolated a gram-positive organism in the majority of cases. Improvement in serum creatinine suggests good prognosis. The mortality in ARF and non-ARF groups is similar.
Sarcoidosis is being increasingly recognised in Kuwait. Twenty patients were studied over three years and the clinical, biochemical and radiological data were analysed. The clinical profile revealed thoracic involvement in all the patients as well as constitutional symptoms (50%), arthralgia (55%), arthritis (15%), chest infection (35%), tuberculosis (10%), hypercalcaemia (5%), angina (15%) and hypertension (20%). None had central nervous system manifestations. Other clinical signs were erythema nodosum (25%), hepatomegaly (30%) splenomegaly (15%) and chest signs (25%), together with salivary gland (15%), skin (15%), eye (15%), and cardiac involvement (5%). The tuberculin test was negative in all those tested. The patients were classified radiologically into stage I (55%), stage II (40%) and stage III (5%) of the disease. The clinical profile was similar to the Western pattern of the disease, but there were several differences including an older age group, more frequent constitutional symptoms, the rarity of ocular and central nervous system involvement, and initial presentation as a chest infection. Therapy with steroids alone or steroids and azathioprine was used when appropriate and the response to therapy monitored.
Severe asthma and diabetes have been reported not to co-exist in the same patient. Various studies have attributed this to the possible association of asthma with hyperinsulinism, increased responsiveness to insulin or to beta-blockade. Previous studies have not addressed all these possible mechanisms in the same patient. In this prospective study, 7 atopic asthmatics and 7 age and sex-matched healthy controls underwent glucose, insulin and glucagon tolerance tests. The results showed no evidence of hyperinsulinism or increased responsiveness to insulin. Intravenous administration of glucagon, however, showed a lesser increase of glucose and insulin in asthmatics. Since glucagon has a beta-agonist effect on the liver and activates glycogenolysis and gluconeogenesis via beta-receptor stimulation and stimulates insulin secretion by activating adenylate cyclase of pancreatic beta-cells through beta-receptors, the results of glucagon tolerance test in our study may therefore suggest the presence of partial beta-blockade in atopic asthmatics.
A most unusual case of Munchausen's syndrome in an 18-year-old Yemeni female is described. The patient presented with bleeding from various sites; repeated subcutaneous emphysema of the face, orbit and upper chest; ulcers on the tongue, and dermatitis autogenica. The illness was confirmed to be factitious and self-induced when she was caught red-handed trying to inject air. We believe this to be the first report of a case where all these features occurred concurrently and only the second report where a patient developed self-induced orbital emphysema.
A 17-year-old patient was found to have cyanosis. A right-to-left shunt was suspected clinically and confirmed by noninvasive techniques such as perfusion lung scan and contrast echocardiography. Angiography showed this shunt to be between the left pulmonary artery and the left atrium. We believe this to be the first report of a hitherto undescribed congenital anomaly.
A patient with severe chronic asthma, who had become refractory to conventional antiasthma drugs, is described. As a last resort, therapeutic bronchoalveolar lavage was performed using beta-stimulant, steroid, and mucolytic solutions. The patient, who had had asthmatic attacks almost every day prior to lavage, showed a dramatic and sustained improvement in his condition. It is suggested that therapeutic bronchoalveolar lavage may be useful in chronic asthmatics who have become refractory to conventional treatment because of mucus plugging of their bronchi.
Pulmonary infection caused by Arachnia propionica in a patient with hairy cell leukaemia is described. The organism is highly sensitive to penicillin but the patient succumbed to his illness despite treatment with penicillin and various other antibiotics. Various types of opportunist infections are common in patients with hairy cell leukaemia but we believe this to be the first report of infection by Arachnia propionica in such a patient.
Recurrent hereditary polyserositis (RHP) or familial Mediterranean fever (FMF) is a chronic inherited illness of obscure aetiology. The disease is characterised by paroxysmal attacks of fever, peritonitis, pleuritis or arthritis, and predominantly affects Sephardic Jews, Arabs, Turks and Armenians. In this study, we report our 11-year experience of 175 Arab patients with this disease. As with other ethnic groups, the most common manifestation (93.7 per cent) was peritonitis. Arthritis (33.7 per cent) and pleurisy (32 per cent) were next in frequency. Adult patients in this series unlike those in other ethnic groups, rarely presented with arthritis. Similarly rare were amyloidosis, rashes, splenomegaly, hepatomegaly or lymphadenopathy. The aetiology of this disease is not clear but we suspect that abnormalities in catecholamine metabolism may be a factor in the pathogenesis.
Optic neuritis, a well known adverse effect of ethambutol, is related to the dose and duration of the therapy. The patient described here developed rapidly progressive deterioration of vision after only 3 days of treatment with ethambutol. Such a case has not been reported before and it is suspected that this was an idiosyncratic reaction.
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A case of hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu disease) is described who presented with severe, central chest pain mimicking acute myocardial infarction, a presentation which has not been described before. He was found to have developed spontaneous haemothorax which is a very rare complication of this disease.
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